Abstract

Although most non-melanoma skin cancers are felt to be sporadic in origin, these tumors do play a role in several cancer predisposition syndromes. The manifestations of skin cancers in these hereditary populations can include diagnosis at extremely early ages and/or multiple primary cancers, as well as tumors at less common sites. Awareness of baseline skin cancer risks for these individuals is important, particularly in the setting of treatments that may compromise the immune system and further increase risk of cutaneous malignancies. Additionally, diagnosis of these disorders and management of non-cutaneous manifestations of these diseases have profound implications for both the patient and their family. This review highlights the current literature on the diagnosis, features, and non-melanoma skin cancer risks associated with lesser-known cancer predisposition syndromes, including bone marrow failure disorders, genomic instability disorders, and base excision repair disorders.

Highlights

  • Frontiers in OncologyMost non-melanoma skin cancers are felt to be sporadic in origin, these tumors do play a role in several cancer predisposition syndromes

  • Non-melanoma skin cancers are very common, but their hereditary risk factors receive little attention

  • This review will synthesize the current literature on non-melanoma skin cancer risk in the following cancer predisposition syndromes: Telomere Biology Disorders, Fanconi anemia, Xeroderma pigmentosum, Bloom syndrome, Werner syndrome, Rothmund-Thompson syndrome, and Ferguson-Smith syndrome

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Summary

Frontiers in Oncology

Most non-melanoma skin cancers are felt to be sporadic in origin, these tumors do play a role in several cancer predisposition syndromes. Awareness of baseline skin cancer risks for these individuals is important, in the setting of treatments that may compromise the immune system and further increase risk of cutaneous malignancies. Diagnosis of these disorders and management of non-cutaneous manifestations of these diseases have profound implications for both the patient and their family. This review highlights the current literature on the diagnosis, features, and non-melanoma skin cancer risks associated with lesser-known cancer predisposition syndromes, including bone marrow failure disorders, genomic instability disorders, and base excision repair disorders

INTRODUCTION
Hereditary Predispositions and Cutaneous Risk
BONE MARROW FAILURE SYNDROMES
TELOMERE BIOLOGY DISORDERS
Means of Diagnosis
Incidence of Skin Cancer
FANCONI ANEMIA
GENOMIC INSTABILITY SYNDROMES
BLOOM SYNDROME
WERNER SYNDROME
ROTHMUND THOMSON SYNDROME
XERODERMA PIGMENTOSUM
Inheritance Percent of syndrome associated pattern with PV in gene
AR AR AR AR AR AR AR AR AR
Telomere Biology Disorders Fanconi Anemia
Financial support available
Findings
CONCLUSION
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